Waldenström’s macroglobulinemia

Abstract

The diagnostic concept of Waldenström’s macroglobulinemia (WM) has changed dramatically since Jan Waldenström originally reported two patients with a syndrome of oronasal bleeding, lymphadenopathy, an elevated sedimentation rate, hyperviscosity, normal bone films, cytopenias, and a bone marrow with a predominantly bone marrow infiltrate (Waldenström 1944). The second international workshop on WM attempted to refine the working definition of the disease within the context of a LPL (Owen et al. 2003a). Waldenström’s macroglobulinemia (WM) is a distinct clinicopathological entity resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphocytes, lymphoplasmacytic cells, and plasma cells which secrete a monoclonal IgM protein. This condition is considered to correspond to the lymphoplasmacytic lymphoma (LPL) as defined by the World Health Organization classification system (Swerdlow et al. 2008). Most cases of LPL are WM, with less than 5 % of cases made up of IgA, IgG, and nonsecreting LPL.